• Clinical and Experimental Pediatrics
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• 제59권2호
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• pp.59-64
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• 2016

Purpose: Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea. Methods: Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. Results: Their median age at the time of diagnosis was 2.5 months (range, 0-20 months). The median follow-up period was 25.5 months (range, 0-94 months). The median length at birth was 50.0 cm (range, 48-53 cm); however, height became more prominent over time, and the patients were taller than the 97th percentile at the time of the study. None of the patients had any relevant family history. Four of the 5 patients who underwent DNA sequencing had a fibrillin 1 gene mutation. All the patients with echocardiographic data of the aortic root had a z score of >2. All had mitral and tricuspid valve prolapse, and various degrees of mitral and tricuspid regurgitation. Five patients underwent open-heart surgery, including mitral valve replacement, of whom two required multiple operations. The median age at mitral valve replacement was 28.5 months (range, 5-69 months). Seven patients showed congestive heart failure before surgery or during follow-up, and required multiple anti-heart failure medications. Four patients died of heart failure at a median age of 12 months. Conclusion: The prognosis of infantile MFS is poor; thus, early diagnosis and timely cautious treatment are essential to prevent further morbidity and mortality.

• Journal of Chest Surgery
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• 제21권5호
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• pp.893-898
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• 1988

A number of centers have recorded a significant incidence of primary tissue valve failure with the Ionescu-Shiley pericardial valve. Clinically, Endothelialization and host tissue ingrowth on the cloth and the leaflets at the edge of the frame greatly reduced the amounts of abrasion and the incidence of tissue failure. In most cases severe regurgitation was caused by leaflet tears adjacent to the edge of the cloth-covered stent. We report a case of spontaneous disruption of one cusp on the Ionescu-Shiley pericardial xenograft in mitral position at 6years and its successful management.

• Journal of Chest Surgery
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• 제38권2호
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• pp.132-138
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• 2005

배경: 승모판막 성형술은 승모판막 폐쇄부전증에서 적절한 치료이지만 승모판막 폐쇄부전의 재발과 그에 따른 재수술이 제한점이다. 본 연구는 승모판막 성형술 후 재발의 원인에 대해 술기와 판막 요인에 대하여 분석하였다 대상 및 방법: 1994년 1월부터 2002년 1월까지 승모판막 폐쇄부전증으로 승모판막 성형술을 받은 493명을 대상으로 하였다. 승모판막 폐쇄부전의 원인으로는 퇴행성이 252예$(51.5\%)$, 류마티스성이 156예$(31.6\%)$, 다른 원인이 85예$(16.9\%)$였다. 성형술은 링을 사용한 판막륜성형술이 446예$(90.5\%)$, 신건삭 재건술이 227예$(46\%)$, 사각 절제술이 125예$(25.3\%)$, 건삭 전이술이 28예 $(5.7\%)$, Alfieri 방법이 8예$(1.6\%)$였다. 평균 추적 관찰 기간은 $29.04\pm22.81$개월이었다. 결과: 5예의 조기 사망$(1.01\%)$과 5예의 만기 사망$(1.01\%)$이 있었다. 재수술율은 $1.42\%$였다. 승모판막 폐쇄부전의 재발은 45예$(9.1\%)$에서 있었다. 24예에서 술기와 동반된 재발이 발생하였는데, 불완전한 성형술로 인한 재발이 14예, 신건삭 길이의 부적합으로 인한 재발이 8예, 기타 원인으로 인한 재발이 2예 있었다. 21예에서 판막 요인에 의한 재발이 발생하였는데, 류마티스성 질병 진행으로 인한 재발이 10예, 건삭의 연장이나 탈출로 인한 재발이 5예, 기타 원인으로 인한 재발이 6예 있었다. 심한 승모판막 폐쇄 부전은 불완전한 성형술 후 많이 나타났으며(p<0.001),판막 요인에 의한 재발은 류마티스성 진행과 강한 상관관계를 보였다(p<0.05). 결론: 승모판막 성형술의 내구성을 결정하는 위험 인자로 수술의 완전성이 가장 중요하므로, 초기 성형술 후 수슬장에서 경식도 초음파로 검사하는 것이 필수적이다.

• Journal of Korean Neurosurgical Society
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• 제30권1호
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• pp.73-77
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• 2001

Object : To determine whether to use surgical or medical therapy in treatment of infectious intracranial aneurysms, we reviewed two recent cases of infectious intracranial aneurysms and others known previous reports of aforementioned cases. Hence, we attempted to compare the validity and effectiveness of surgical and medical treatment. Method : Recently, we treated two cases of ruptured infectious intracranial aneurysms. In former case, the aneurysm was located distal to the middle cerebral artery in a patient with mild mitral regurgitation of the heart. In latter case, the aneurysm was multiple with varying hemorrhage. The hemorrhage was located bilaterally and a moderate mitral regurgitation and infective endocarditis were accompanied in this patient. Result : Due to the large size of the intracranial hematoma, stable medical condition, and easy resectability, we treated the former patient surgically. And, because of successive hemorrhage by multiple aneurysmal rupture, and the risk of heart failure, we treated the latter patient medically with serial follow-up angiography. Both patients are at present in good health. Conclusion : Because of the variability in associated factors, such as the patient's health, the number of lesions, location, anatomy of the aneurysms and the causative organism, each patient's care must be individualized and tailored to the patient's particular clinical situation.

• Journal of Chest Surgery
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• 제48권6호
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• pp.407-410
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• 2015

A two-month-old infant presented with coarctation of the aorta, severe left ventricular dysfunction, and moderate to severe mitral regurgitation. Through median sternotomy, the aortic arch was repaired under cardiopulmonary bypass and regional cerebral perfusion. The patient was postoperatively supported with a left ventricular assist device for five days. Left ventricular function gradually improved, eventually recovering with the concomitant regression of mitral regurgitation. Prompt surgical repair of coarctation of the aorta is indicated for patients with severe left ventricular dysfunction. A central approach for surgical repair with a back-up left ventricular assist device is a safe and effective treatment strategy for these patients.

• Journal of Chest Surgery
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• 제52권1호
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• pp.9-15
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• 2019

Background: Although aortic valve repair can reduce prosthesis-related complications, rheumatic aortic regurgitation (AR) caused by leaflet restriction is a significant risk factor for recurrent AR. In this study, we evaluated the long-term results of the leaflet extension technique for rheumatic AR. Methods: Between 1995 and 2016, 33 patients underwent aortic valve repair using the leaflet extension technique with autologous pericardium for rheumatic pure AR. Twenty patients had severe AR and 9 had combined moderate or greater mitral regurgitation. Their mean age was $32.2{\pm}13.9$ years. The mean follow-up duration was $18.3{\pm}5.8$ years. Results: There were no cases of operative mortality, but postoperative complications occurred in 5 patients. Overall survival at 10 and 20 years was 93.5% and 87.1%, respectively. There were no thromboembolic cerebrovascular events, but 4 late deaths occurred, as well as a bleeding event in 1 patient who was taking warfarin. Twelve patients underwent aortic valve reoperation. The mean interval to reoperation was $13.1{\pm}6.1$ years. Freedom from reoperation at 10 and 20 years was 96.7% and 66.6%, respectively. Conclusion: The long-term results of the leaflet extension technique showed acceptable durability and a low incidence of thromboembolic events and bleeding. The leaflet extension technique may be a good option for young patients with rheumatic AR.

• Journal of Chest Surgery
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• 제55권1호
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• pp.69-76
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• 2022

Background: While the use of bioprosthetic valves for mitral valve replacement (MVR) is increasing, very few studies have compared bovine pericardial and porcine valves in the mitral position to help guide bioprosthetic selection. Methods: In the present study, patients who underwent MVR using bovine pericardial valves were compared with those who underwent MVR with porcine bioprostheses between January 1996 and July 2018. Those with prior MVR, infective endocarditis, congenital mitral valve disease, or ischemic mitral regurgitation were excluded. The primary outcomes were structural valve deterioration (SVD) and mitral valve reoperation from any cause, and death was regarded as a competing risk. Competing risk analysis and propensity score-matching were used for comparisons. Results: Among the 388 patients enrolled, pericardial and porcine bioprostheses were implanted in 217 (55.9%) and 171 (44.1%), respectively. Propensity score-matching yielded 122 pairs of patients that were well-balanced for all baseline covariates. No significant differences were observed between the groups in unadjusted (p=0.09) and adjusted overall survival (hazard ratio [HR], 1.13; 95% confidence interval [CI], 0.72-1.76; p=0.60). Competing risk analysis revealed no significant differences in the risks of mitral reoperation (HR, 1.07; 95% CI, 0.50-2.27; p=0.86) and development of SVD (HR, 1.57; 95% CI, 0.56-4.36; p=0.39) between the groups. Matched population analysis confirmed similar results regarding reoperation (HR, 0.99; 95% CI, 0.40-3.22; p=0.98) and SVD (HR, 1.39; 95% CI, 0.41-4.73; p=0.60). Conclusion: No significant differences in survival or valve durability were observed between bovine pericardial and porcine bioprosthetic MVR. These findings require further validation through studies with larger sample sizes.

• Journal of Chest Surgery
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• 제15권1호
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• pp.53-60
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• 1982

M-mode echocardiographic studies of left ventricular function in mitral valve disease were evaluated in 19 surgically treated patients before and one year after operation (mean 12.7 months). Twelve patients had mitral stenosis (MS) and seven patients had mitral regurgitation (MR). Before surgery, average end-diastolic and end systolic dimensions (EDD, and ESD) and left atrial dimension were significantly greater than normal in subject with MR. After surgery, EDD fell significantly from $66.5{\pm}8.4$ (SD)mm to $52.7{\pm}6.3$mm (P 0.01) at the time of late follow up study; ESD fell significantly from $46.5{\pm}9.7$mm to $36.4{\pm}8.6$ (P 0.05) on early follow up study; left atrial dimension fell significantly from $60.5{\pm}6.8$mm to $48.1{\pm}7.2$mm (P 0.01) at the time of the late follow up study. In patients with MS, EDD and ESD were normal and did not change significantly at any time after surgery. The left ventricular ejection fraction (E.F.) was normal in both groups. preoperatively([MR: $64.2{\pm}15.1$, MS: $65.7{\pm}12.3$). After surgery, E.F. did not change significantly at any time after surgery in both groups, but de-creased from $64.2{\pm}15.1$% to $59.5{\pm}11.2$% in MR patients at the time of early follow up study.

• Journal of Chest Surgery
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• 제42권2호
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• pp.175-183
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• 2009

배경: 소아에서 승모판막 기형은 드물며 각 기관마다 비교적 작고 제한된 경험들을 가지고 있다. 또한 소아에서는 승모판막 치환술은 성장 후를 고려해야 하는 문제점이 있다. 본 연구에서는 소아에서 승모판막 성형술을 시행한 환자에서의 성적을 분석하고자 하였다. 대상 및 방법: 1996년부터 2005년까지 승모판막 성형술을 시행한 총 64명의 환자를 대상으로 하였다. 수술 당시 평균 연령은 $5.5{\pm}4.7$세였고, 남자는 28명, 여자는 36명이었다. 환자는 승모판막 기형과 동반질환에 따라 두 군으로 나누어 분석하였다. 1군은 승모판막 기형만 있거나, 심방중격결손이나 동맥관 개존증을 동반하고 있는 경우로 34명(53.1%), 2군은 이외 다른 선천성 심기형을 같이 동반하고 있는 경우로 30명(46.9%)이었다. 결과: 수술 후 재원기간 내 전체사망률은 6.3%로, 1군에서 2명(5.9%), 2군에서 3명(10%)으로 각 군간에 의미 있는 차이는 없었다. 수술 후 합병증 발생률은 18.8%로, 1군에서 5명(14.7%), 2군에서 7명(23.3%)으로 각 군 간에 의미 있는 차이는 없었다. 중간 추적관찰기간은 4.6년이었다(범위 $0.5{\sim}12.2$년). 10년 생존율은 95.3%였고 10명에서 재수술이 필요하였다. 이 중 2명은 잔여 승모판막 폐쇄부전으로 재원기간 중 재수술하였고 10명 중 7명에서 승모판막 치환술을 시행하였다. 10년 무재수술 생존률은 76.1%였다. 육안적 소견에서 가장 많은 기형은 판막륜 확장과 판막 탈출이었고, 성형술은 각 환자에서 평균 $2.1{\pm}1.1$개의 술식이 시행되었다. 수술 직후 재원기간 중 시행한 심초음파 추적결과에서는, 승모판막 폐쇄부전은 의미 있게 개선되었고, 추적 관찰 중 시행한 심초음파 결과에서도 수술 1년, 5년, 10년 후 시행한 심초음파 결과에서 승모판막 역류는 통계학적으로 유의하게 개선되었다. 한편 승모판막 역류로 수술 받은 환자의 32.8%에서 추적 관찰 중 판막 협착의 소견을 보였으며, 수술술식과 판막 협착의 발생 관계도 통계학적으로 유의한 소견을 보였다. 결론: 선천성 승모판막 기형을 가진 환자에서 승모판막 성형술을 시행한 경우 1군과 2군간의 결과에 통계학적으로 유의한 차이가 없었고, 두 군에서 모두 비교적 양호한 성적을 보였다. 또한 소아에서 승모판막 성형술에 대한 심초음파 결과도 양호함을 객관적으로 증명하였으나, 선천성 승모판막 기형의 수술 후 장기적 경과 관찰에서 발견되는 승모판막 협착이나 역류를 조기에 발견하기 위한 정기적인 경과 관찰이 필요하다.

• Journal of Chest Surgery
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• 제40권12호
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• pp.855-858
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• 2007

승모판막 치환술 후 발생하는 좌심실파열은 치명적인 합병증이다. 30년 전 국외에서 승모판막에 대한 수술을 받은 병력이 있는 54세 여자 환자가 심한 승모판막 협착증과 심한 삼첨판막 폐쇄부전을 주소로 내원하여 승모판막 치환술 및 삼첨판막륜 성형술을 시행받았다. 심폐기 이탈 후 수술장 내에서 발생한 대량출혈로 심폐기 재가동 상태에서 평가한 결과 좌심방으로부터 3 cm 후방부위의 방실 골짜기에서 발생한 급성 제I형 좌심실 파열로 진단하고 심외막 접근법으로 테프론 펠트를 이용한 일차 봉합술, 심막첨포를 이용한 밀봉 봉합술 및 피브린 접착제를 적용하여 성공적으로 봉합하였으며 환자는 수술 14일째 합병증 없이 퇴원하였다. 이에 승모판막 치환술 후 발생하는 제I형 좌심실 파열에 대한 문헌고찰과 함께 증례를 보고하고자 한다.